Two cases of congenital pyloric atresia (CPA) are, hereby, reported. One was suspected on antenatal ultrasound and turned out to be an isolated anomaly. Other patient had a rare association of aplasia cutis congenita with congenital pyloric atresia. The lesions of aplasia cutis congenita were multiple while congenital pyloric atresia was of type II. The patient with an isolated lesion survived following surgery while the other baby died of sepsis in postoperative period.